C Gnanamuthu

Clinical and laboratory features and response to treatment in patients presenting with vitamin B12 deficiency-related neurological syndromes.

AIMS AND OBJECTIVES To study the clinical and laboratory features of patients admitted with vitamin B12 deficiency-related (B12def) neurological syndromes. SETTINGS AND DESIGN A hospital-based retrospective and prospective study conducted at a referral teaching hospital. MATERIALS AND METHODS Consecutive patients admitted with vitamin B12 deficiency-related neurological disorders during a three-year period from June 2000 to May 2003 were included. Data regarding clinical and laboratory features were obtained. Follow-up was done at least six months following treatment with parenteral vitamin B12. Chi-square test was used for statistical analysis. RESULTS A total of 63 patients (52 males) with a mean age of 46.2 years were studied. The mean duration of symptoms at presentation was 10.3 months. Myeloneuropathy (54%) was the commonest neurological manifestation, followed by myeloneuropathy with cognitive dysfunction (34%), and peripheral neuropathy (9%). Neuropsychiatric manifestations and dementia were observed in 38% and 19% of patients respectively. All the patients had megaloblastic changes in the bone marrow smear. Eleven (17.5%) patients had both hemoglobin and the mean corpuscular volume (MCV) within the normal range. Follow-up after at least six months of therapy with parenteral B12 showed improvement in 54% patients. CONCLUSIONS A high index of suspicion of B12def is required in patients presenting with myelopathy, cognitive decline, or neuropathy. A normal hemoglobin or MCV does not exclude B12def; therefore, other tests such as bone marrow smear and serum vitamin B12 assay are essential, as the condition is often reversible with treatment.

Behaviour problems in children and adolescents with seizure disorder: associations and risk factors

PROBLEM Fifty million people worldwide have epilepsy of whom 33 million are children. Children and adolescents with epilepsy are known to have high rates of behaviour problems and psychiatric co-morbidity. No studies on this topic have been conducted in developing countries, although 90% of such children live in the developing world. METHODS The current study, set in Vellore in India, assessed the prevalence of psychopathology and its associations in children and adolescents with seizure disorder. One hundred and thirty two children were rated for psychopathology on the Child Behaviours Check List, and those who qualified as psychiatric cases were compared with those who did not. RESULTS Seizure disorder was associated with significant psychopathology in 53.8% children. Belonging to a higher income group and living in an urban area (OR: 7.61, 95% CI: 2.78-20.8, p=0.0001), having longer than 3 years of illness (OR: 2.39, 95% CI: 2.18-5.67, p=0.03) and being treated by more than one anti-epileptic drug (OR: 3.08, 95% CI: 1.09-8.72, p=0.03) independently predicted psychopathology. CONCLUSIONS Psychopathology reflected a complex interaction of seizures, socio-demographic factors and treatment related variables, in accord with studies in industrialised countries. Clinicians should be sensitive to identifying psychopathology since this may lead to effective interventions for this group of children.

Postinfectious pandysautonomia with complete recovery after intravenous immunoglobulin therapy

Acute dysfunction of the autonomic nervous system was first reported as severe parasympathetic and sympathetic failure without other neurologic manifestations by Young et al.1 in 1969. Since then, several other cases have been described.2-6 The characteristic features of this syndrome include severe orthostatic hypotension, impaired gastrointestinal and bladder motility, dry mucous membranes, and loss of sweating ability with impaired pupillary light reflex and accommodation reflex. These features are similar to those seen in Guillain-Barre syndrome (GBS) accompanied by autonomic disturbances. However, in pandysautonomia, there is no clinical or neurophysiological evidence for involvement of the somatic nerve fibers. Acute pandysautonomia is increasingly recognized as a possible variant of GBS. The presence of raised CSF protein and varying degrees of sensory loss and dysesthesias2,3 lends credence to this belief. IV immunoglobulin (Ig) is of benefit in GBS,7 and it seems reasonable to expect that pandysautonomia should also show a favorable response. …

Cranial nerve involvement in patients with leprous neuropathy.

BACKGROUND Leprosy is one of the most common causes of peripheral neuropathy, perhaps closely matched by diabetic neuropathy. Patterns of peripheral neuropathy in leprosy can be varied, which may include mononeuropathy, mononeuritis multiplex and symmetric polyneuropathy. Cranial nerves, especially facial and trigeminal nerves, are also commonly involved in leprosy. AIMS To find out the pattern and spectrum of cranial nerve involvement in a consecutive series of patients with leprous neuropathy. SETTINGS AND DESIGN A retrospective review of patients admitted with leprosy to the Neurology Department of a tertiary care center. MATERIALS AND METHODS All consecutive patients admitted during an 8-year period (1995-2003) and diagnosed to have leprosy were included. They were clinically evaluated to determine the frequency and pattern of cranial nerve involvement. RESULTS About 18% (9/51) of the leprosy patients seen during that period had clinical evidence of cranial nerve involvement. Facial and trigeminal nerves were the most commonly affected (five and four patients respectively). CONCLUSIONS Cranial nerve involvement is common in leprosy, which emphasizes the need to carefully examine them. Also, one should exclude leprosy in patients presenting with isolated cranial neuropathies.

Stiff-Man Syndrome in Childhood

Stiff-man syndrome is a disease of sporadic occurrence. An autoimmune pathogenesis is thought to involve the spinal interneuronal circuits, resulting in isolation of the motor neurons from their inhibitory influence1. Most of the reported cases have been in adults.

Human cerebrospinal fluid acetylcholinesterase and butyrylcholinesterase. Evidence for identity between the serum and cerebrospinal fluid butyrylcholinesterase

Human cerebrospinal fluid contained both acetylcholinesterase (EC 3.1.1.7) and butyrylcholinesterase (EC 3.1.1.8) and they were estimated in the presence of selective inhibitors. Butyrylcholinesterase of human cerebrospinal fluid was similar to human serum butyrylcholinesterase in its electrophoretic mobility, glycoprotein nature and tyramine activation of the aryl acylamidase (EC 3.5.1.13) activity exhibited by butyrylcholinesterase. Moreover antibody raised against human serum purified butyrylcholinesterase could completely immunoprecipitate butyrylcholinesterase from human cerebrospinal fluid without affecting acetylcholinesterase. It is suggested that a useful method for the precise determination of acetylcholinesterase in human cerebrospinal fluid would be removal of butyrylcholinesterase by immunoprecipitation using antibody raised against human serum butyrylcholinesterase.

Cervical spine injuries in 64 attempted suicidal hangings in India.

This report describes the outcome of 64 attempted suicidal hangings, 81% of whom made a full recovery.